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  • Adult Niemann-Pick disease  (1)
  • Amyloid  (1)
  • Caecal perforation  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 399 (1983), S. 233-236 
    ISSN: 1432-2307
    Keywords: Amyloid ; Amyloidosis ; Lymph nodes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of a patient presenting with supraclavicular and cervical lymph node enlargement with demonstration of amyloid is presented. Histologic features are described. Amyloid deposition was not found in any other organ. Immunohistochemical typing with antisera raised against protein AA, Aλ, and Aκ showed a reaction only against Aλ. Thus, this case belongs to the immunoglobulin λ light chain-derived types that occur in many other parts of the body, particularly the respiratory tract.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 419 (1991), S. 349-353 
    ISSN: 1432-2307
    Keywords: Long-term haemodialysis ; β 2-Microglobulin ; Derived amyloidosis ; Caecal perforation ; Pathological hip fracture ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 73-year-old man with chronic renal failure of undetermined aetiology had received haemodialysis for 12 years when he died of acute purulent peritonitis due to caecal perforation. Amyloid deposits detected in a cystic bone lesion in the left hip had caused a pathological fracture 17 days before death. At autopsy, extensive amyloid deposits were found in the osteoarticular system, in the cartilaginous surface and the capsular tissue of joints, ligaments, vertebral discs and bone. In addition, vascular amyloid deposits were diagnosed in the heart, kidneys, testes, lungs, skin and in the gastrointestinal tract. A special feature of this case were interstitial amyloid deposits forming a fine-meshed structure in the myocardium and plate-like deposits in the gastrointestinal tract. Immunohistochemically, all these deposits reacted strongly with antibody to humanβ 2-microglobulin but showed no reaction with antibodies to AA, Alambda, A-kappa and AF. The present case demonstrates that extra-osteoarticular manifestations of AB-amyloidosis can cause serious complications.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-2307
    Keywords: Adult Niemann-Pick disease ; Generalized AL-amyloidosis ; Progressive liver failure ; Fibroblast culture ; Immunohistochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a case in which an adult form of Niemann-Pick disease (type B of NPD) was associated with a rapidly progressive generalized AL amyloidosis of kappa type. Both diagnosis were made by biopsy, the NPD by bone marrow biopsy and fibroblast culture, the amyloidosis by liver biopsy. Malignant non-Hodgkin lymphoma was not found. The patient, a 67-year-old woman, died from hepatic coma subsequent to a progressive liver failure. We discuss possible relations between the lysosomal storage disease and the development and rapid progression of amyloidosis.
    Type of Medium: Electronic Resource
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